haem Flashcards by Fardowsa Mohamed

henoch - purpura?

Henoch-Schonlein Purpura (HSP )= small vessel vasculitis caused by immune complex composite from infection triggers

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triggers/ etiology for HP?

genetic predosipition, preceding infection (group A strep 1-3 wks before) & genetic pre-disposition, drugs, vaccines ,GI infection some antibodies (

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pathophysiology of HP?

igA complex -> aggregate and deposit in target organs post URT infection.

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CF of HSP?

palpable purpura, arthritis/arthralgia/ GI or renal sx.
-hematuria/ proteinuria. ARF/CRF
-rash over buttocks & extensor surface.
abdominal pain due to mesenteric vasculitis.

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dx ? dd

autoimmune markers, fbc, high esr, cbc , platelet counts.
ddx: acute abdomen, renal syndromes, SLE, IBD, RA

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treatment of hsp

immunosuppressive agent or self limited.
- iv fluids
iv prednisolone if severe esp if renal function compromised.
NSAId 1st line

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DIC:

activation of coagulation syst6em caused via marked activation of fibrinolytic cascade.

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acute and chronic cause?

acute: sepsis, obstetric shock, burns, hemorrhages, infections, acute leukemias., acute hemolytic transfusion reaction.

chronic: gastric/pancreatic Ca.

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stages/ phases of DIC?

hypercoagulation: activation and overuse of the coagulation system.
-exhaustion due to formation of thrombi.
decrease in serum platelets. thrombocytopenia.
final: resolve, complication or death.
hyper vs hypocoagulation in thrombi formation in capillaries.

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pathophysiology of DIC?

damaged tissue release tissue factor.
activate fibrinogen cascade and formation of micro-vessel thrombi.
3.reduction in conc of fibrin factors. impaired hemostatic status.
fibrinolytic activity: increase d-dimer activity.
5.FDP’s inhibit coagulatin factors and increase exporesison of thrombomodulin-thrombi complex in blood (diagnostic) activate protein c. inactivates factor 5a & 8a

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acute vs chronic DIC?

acute: purpura, ecchymosis, bleeds in body orifices. digital gangrene and adult ARDS.
chronic: hemorrhagic crisis or asymtpomatic

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DIAGNOSITC OF dic?

decrease fibrinogern activity, prolonged PT, APTT.
increase d-dimer,
generalised thrombocytopenia.

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treatment:

heparin for acute: decrease fibrinogen-fibrin conversion. shuts off formation of thrombi.
fresh platelet plasma infusion
-LMW herparin for chronic: chronic embolism formation in metastasis.
warfarin: unsuccessful.
trx underlying cause and resect any tumors, radio/ chemo and immunosuppression.

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MM
+ triad of abnormality

malignant proliferation of plasma cell’s in BM. triad of abnormalities:
1. accumulation of b-cells
2.punmched out lesions in Bone.
3. monoclonal aggregation of igG (secreted by plasma cells)

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etiology & pathophysiology

no known genetic component or environmental factors (idiopathic) affects >60. BM cells stromal interact with cancerous cells and adhesive. CK , drug resisANCE AND GROWTH.
ck released and harms body. iL-3 inhibit osteoblast progenitor. OPG inhibited via DKK1 and inhibits osteoclast. osteoclast can stimulate via IL-6 amplification of MM. hypercalcemia-> nerve issues & dheydration.

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clinical features

backpain, fractures, URTI, anaemia, RF, proteinuria, dehydration. scans show lytic bone lesions.
bone marrow< 15 % normally. MM shifts hemopoietic stem cell-> abnormal Ab (just light chain-paraportein; igG and igA).

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diagnostic

light chain -bens johns proteins. shift of haemopoietin SC shift from myeloid to lymphoid proliferation. increase n paraprotein, decrease in normal AB, urea/nitrogen, hypercalcemia, anaemia, increase in creatinine. CT/X ray shows bone changes. bone profile test. bone aspiration and bone marrow biopsy. >10% monoclonal plasma cell in BM, Ab urine/ serum.

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mneomic (CRAB)+ extra details:

Calcaemic Renal failure Anaemia Bone lesions.

Bone pain with hypercalcemia 🡪 neoplastic pl cells activate RANK receptor
on osteoclasts = bone destruction. “punched out” lesions seen on x-ray - ↑ risk of fracture
↑ risk of infx 🡪 monoclonal ab lacks Ag diversity; infx most common cause of death
10 AL amyloidosis = free light chains circulate in serum + deposit in tissues
Proteinuria = free light chain excreted in urine as Bence Jones proteins 🡪 ↑ risk of renal failure

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trx:

chemo, radiation.
standard: melphalan, prednisolone (paraprotein and bone degradation reduce).
fitter pts: high dose therapy, stem cell rescue and VAD (vincristine, Adriamycin and dexamethasone) used to treat most cancers

staging

3 staGES if the B2M count serum is above 5.5 then the survival rate reduces. & serum albumin (assoc with IL-6-> encourages growth of MM) serum/urine electrophoresis

hemaotpoesis normal

pluripotent (multipotent-BM).
myeloid (myeoblast, proerythroblast), monoblast and megakaryoblast).
& lymphoid cells (t and B lymphocytes).
proerythroblast-> normoblast-> reticulocytes-> erythrocytes (120 days)
myeloblast-> promeyloytes-> myelocytes-> metamelacyocyes (granulocytes- Neu, baso, esino) 7 hrsin blood
mooblast-> promonoblat-> monocytes.
mekaryoblast-> promegakaryocites-> megakaroyctes-> platelets (7 day life)
only and lymphoid granulocytes an monocyte have nucleus.
haemostasis 1st .
neu- bacteiria/ fungus.
baso- histamines
eosino- parasite, allergies.
b-humoral
t-cellular immunity.
yolk sac 3 wks, 6 wks (liver & spleen)-> bm main source.

red marrow-> yellow marrow as we age. inactive until chronic hemolysis.
BM: central skeleton, ribs, pelvis. sternum, vertebrate body-flat bones).
MCV-80-100 fl- av vol of RBC (classify anemia).
low MCV-micro
normocytic
high MCV-macrocytic,
HB-140-180 g/L
females: 120-160
rbc: 4.7-6.1 x 1012
4.2-54 x 1012
ratio of rbc to whole blood (Hct)
40.7-50.3
anemia: low Hct + HB.

pathologies can cause hemopoesis to resume ack in liver+ spleen (extra medullary hemopoietic).

iron defiicency anemia:

developing world: acute GI bleeds, PUD< haemorrhage.
developed: nutritional deficiency,
(female & children -growth excessive prone).
Etiology: low supply (malabsorption) , high demand, loss (hemolyisis).
prematurity, poverty, diet (veg).
pregnacy-lactation, growth.
celiac, and post gastric surgery.
blood loss- colon/cancer, PUD.
intravascular- microangipathic hemlytic anemia, proxysmal nocturia hemolysis.
HB- heme (iron and protophoryin) and globin.
protopyrin increase, low fe2+
microcytic anemia- cells in production-> smaller if not recieving Hb, as hb comes looks bigger relative.
clinical: glossitis, restless leg syndrome, achloryydria-> iron met-> readily resorption.
koilonchyia.
weak, fatgieu, pale, murmur,angina, dyspnoea.

labs: stored in tissue as ferritin.
MCH, HB low, MCV low, HCT low, reticulocytes, wbc normal, platelets icnreased slightly (viscous).
TIBC-> liver more transferrin so increase to catch fe. IL-6 influenes fe2+ if inflammation.
saturation % low since fe2+ low.
RDW: high. becomes mirocytic gadually.
test: biospy (painful).
give patient iron-> if improve (correct dx).
trx: oral ferrous sulfate (melena, CIBH).
(guiac negative)
trx underlying cause if fials consider celiac etc.
IM or IV iron -anaplylaxis (se).

iron deficiency anmeia contnued

13 g, contains 15-20 mg obly 10 g absorbed.
duodenal uptake heme and on heme iron.
cytochrome B-> reduce fe3+ to fe 2+.
stored in interstitial cells.
hepcidin (regulate metabolism and nhibit transport).
hemochromatosis -> liver pathology-> iron overload.
etiology: chelitis (inflammaiton in corner of mouth).
plummer -vinson syndrome- IDA, esophageal web, chelitis.
serum fe2+ high?? in labs.
differentiatonalm: thalassemia, chronic disesaes anemia.
200 mg twice daily.
disease; SE< chronis, RA, TB, high level hepicidin detected.
sideroblastic: x linked by females (only male affected).
acquired ead, drugs, myelodysplasic, myeloid leukemia.
refracotry (doesnt repond to therapy).
ring sideroblast in apperance).
disordered heme ysnthesis , hypochromatic, excess iron.
trx: folic acid, B 6 vitamins (pyroxidine).
cessation of alcohol, drugs etc

pyruvate kinase deficiency

AR, enzyme deficiency-> extravascular hemolysis -> newborn
NO PK, no ATp-> dehydrated RBC, burr cells.
cause oxygen dissoiation curve-> migitats sx of anemia (less severe-> 2,3 BPG).
jaundice, splenomegaly, anemia.
clincal: reticulocyte icnrease. mcv normal or high.
LDH high, UCB high, haptoglobin low.
dilutional anemia in pregnancy-> diagnosed at that time.
trx: rbc infusion, iron and folate, iron chelation (preven secondayr hemochromatosis), splenectomy helps+ vaccines.
BM transplant.
anemia: Hb- 50-100g/L.

G6DP

2 variant: african anf mediatteran variant.
prtoective agent sickle cell, thalassemia, g6dp: malaria.
x linked: only affects males. AR.
fava beans _> cycle halted enzyme faulty
speroxide prevented to fight infeciton
ifneciton preceeds hemolysis the most.
free radical damage to RBC, protect against metahb-HB.
japtobin reduced, stikcs to much->
UC hemolysys- jaundice.
intravascular hemolysiysis
hexose monophate pathway-> no NaDPH.
low Hb,high reticulocytes, high lDH, UC
test G6dp, florescent screening test.
bite peripheral cels. (spleen fiters out oxidised HB)

sickle cell

AS or SS is autosomal recessive. afro caribnea oriign.
protective agent against malaria.
oxygen binds normally to losely and irreversibly.
defect in B globlin gene.
kids -> doesnt happen to infants till 6 months ffrom gamma to beta.
gene mtutation-> missence point mutation.
hydrophiliac-> hydrophobic.
no sickling -> with oxygen but low oxygen means sickling- damage- hemolysis.
cycle starts & worsens.
trx: oxygen. prevention better than cure
right release
polymerised HB- obstruct artery-> veno occlusive crisis.
infection, dehydration, cold, acidosis (related to hypoxia)>
release oxygen faster rhtna normal HB.
3 clinical forms: homozygous (severe-25%)
combined heteroygous-> Hb s+C>
intermediate.
heteroyzgous HbAs sikcle cell).
leg ulcer, infect eyes, abdo, kidneys, cardiac issues, anemia, asplasia, infarcts pumo. mesentery, necrotic BM,

turbid, precipitation for sickle cell-> normal its clear. (solubility in HBs lower).
low Hb,
reticulocyte high, protein electrophoresis.
blood smear

transufse if complciation. oxygen, abx, opds-morphine
stem cell ,avoid high altitiude.
SC trait- hyxodeycarbamide- HBF conc .
vasocclusive sx.

thalassemia alpha

defect in globlin chains.
decrease globlin- low HB-> RBC low.
precipiate, insolunle.
imbalance n productio of chains.
extramedullary and medullary comepnsaiton \9hepatosplenomegaly hyperplasia).
2nd hemochromatois -> iron cheation deferoxamine.
iron fe 2= increases, AR recessive.

protophoryi (siderobalstic anemia-defectie, sideroblast ring).

the more deleted, worser the sx.
2 copies at the loci.
1 locus-> deleted-> asx.
2 loci deletion-> asymptomatic or low MCV anemia
3 loci- hemotetramers (unpaired b chains)-> mderate-> severe anemia-bloods. splenomegaly.
4loci-> bind to gamma, no effecite -electrophoresis-> hydrops fetalis (intrauterine death) Hb barts. accurate test: geentic tests.
loos normall on leectrophoresis.
african , meditterean andsouth east asia

HB H-disease: jaudnice, anmeia, chronic
HA -3 loci
hypochromic anemia. high RDW.
haptoglobin

hemaotpoesis normal

pathologies can cause hemopoesis to resume ack in liver+ spleen (extra medullary hemopoietic).

reticuocytes:

1-2%. < 2% nutritional defiiency.

<100 g/L; pregnant. iron study
tIBC low = low iron-> anemia CKD.
high TIBC-> iron deficency low.
mcirocytic anemia
ferritin high (hide iron).

normocytic anmeiareituclocyte count
<2% hypoproliferation-> leukemia, aplastic anemia-b12 deficiency
ferritin always oppsote of tIBC.
>2% hemolysis
macrocytic:
megaloblastic -b12 deficneyc/ foalte
non megaloblastic-liver/.

macrocytic anemia

headache, angina slow, tired/pale.
big cells100 fl

hypersegmented Neu in megaloblastic.
folate/b12 deficiency
dna synthesis problem (folate deficiency-> nucleus replication issue).
so from reticulocytes-> rbc (size doesnt change).
all cell lines affected due to dna issues.
megaloblast precursor-> haemolysis-> pancytopenia
folate/b12 (THF absorbed , gets ride of CH3 to B12)-> to get catalized-> homocyteine -> methionine.
methocolabine (CH£+ b12).
ileum absortption. autoimune paritetal, chrons.
neurological manifestation ofPNS-> paresthesial, weakness, babinski.
tear drop RBC, high MCV.
beefy red tongu, dry and yellow skin.
extravascular hemolysis-:> LDH high, bilirubin high. homocystein test (defciency in b12).
radiolabelled test: colobamine
trx: hydroxycobolamine 1000 microgram IM for 3 weeks.
folate: poor intake.
neural tube defect in pregnancy. alcohol & cancer reduce folic store

folic aid 5mg (400 mg daiy) for women. 4 months

physiology

biconca,ve n nuclei, ribosome or other organelles, increase SA>
G6DP-< pentose pathway. protect HB against oxidative stress, free radicls.
abnormal fe2+ or methehmoglbon canot transport o2.
HbC-> sickle cell carrier trait .
120 days. 90% extravascular hemolysis.
abornla RBC: electorphoresis.
spherocytes: check Direct coombs tesst.
coombs regent+ donors blood-> positive agglutination
indirect: serums ig (recipitent)+ donors blood0> cooms regent- > positve agglutination.
dct +ve: drugs, autoimmune.
DCT -ve: malaria, hereditary hemolysis spherocytosis (deranged Cell membrane of TBC- problem in protein structure)
fragmentation: blister/ bite cells-> punched out due tooxidative or partial phagocytosis..
g6dp and p.kinase,
fragmentation: MHA, elcampsia, DIC, thrombocytopneia purpura.

haemolytic synd: sx associated w/haemolysis.
hereditaory spherocytosis (AD)-vesicles on RBC
cf: jaundice, anaemia, splenomegaly, splenomegaly, ulcers leg, chronic haemolysis (pigmented stones).
stuck-> hemolysis->
increase urobilnpgen, plasma haptoglobin, hemosiderin in urine.
eyrhtrid hyperplasia of BM.
dx: osmotic fragility-> swell in hypotonic solution (tolerate less)>
fhx genetic
microspherocyte on peripheral blood smear,
coombs +ve.
splenectomy.

chronic myeloid leukemia

9: 22 translocation.
neutrophils, granulcyte, monoblasts.
adult, mature cells (fewer blasts).
smaller cells, dysfunctional.
> 20% acute (blasts).
onset insidious-slow (chronic).
myeloproliferative disorder: polycythemia vera, essential thromocytomeia, MM, CML.
RF: radiation (high dose ioniizng)
age bracket: 45-55. splenomegaly, reactive marrow fibrosis, leukaemia.
ABL and BCR -> fuse together (proco-oncogenes).
reciprocal translocation between chr 9 and 22
good prognosis : CML.
bad prognosis: ALL.
increase proliferation, apoptosis
Trx: tyrosine kinase inhibitor.
CML: fatigue, wt loss, sweating.
generalised, painless, huge splenomegaly(metastasis).
abdo pain, infarction- friction rub.
asx (chronic phase)
accelerated- basophilic, immature.
blast crisis- AML or ALL.
refractory-> death.
lab: peripheral blood smear.
10,000-> 6000
bone marrow -> hypercellular hyperplasia.
cytogenetic-PCR.
basophils increases.
imitinib
second line: dasatinib.

myeoprolfierative neoplasia

CML, PV, ET, Primary myelofibrosis.
-elderly affected.
all cell lines affected.
rapid cell turnover- uric acid- purine metabolism (gout).#
splenomegaly.
atypical megakaryotices-> fibroblasts porliferate.
1.anmyelosis, pancytopenia. initially then drop
hyperviscotiy syndrome
complication: AML,ALL.
jaks 2 activation mutation- epo/TPO.
fibrosis of BM
negative feedback.
visual, neurological signs and bleeds.
clinical: bizzare platelets, hepatosplenomegaly, gout, ldh, alp.
pancytopneia, tear dropcells. anemia, infection.
dry tap on bone marrow.
tr: splenecotmy, steroids-> inhibit fibroblast, allopurinol.
chemo- low dose prednisone, thalidome
definitive: bone transplant.

2nd myelofibrosis P.vera and essential thrombocytosis.

polycthemia vera

mutatin of jaks 2 kinase.
sitmulate receptors of EPO and TPO.
RBC (relative and absolute)
plasma BV decrease. HCT increase.
vera- true (absolute).
primary: mutation, cancer, mutation in erythrocyte receptor mutation.
secondary: inrease EPO (abnormal -inappopriate->RCC, HCC etc.
hypoxia (compensaotry mechanism-appopriate)-. high altitude, cyanotic heart disease.
hyperviscosity syndrome.
bruising (lots of platelets), bleeds -> suck. erythomelagia (red, swellling, tender).
thrombosis.
aquatic pruritis- histamine release (basophile).
management: aspirin, cooling, leg elevation erythromelagia.
CBC dx.
HCT> 48%.
hb> 16 g/L.
check abg for hypoxia as secodary cause.
biopsy-> hypercellularity.
increase everything.
mutation seen.
2 major and 1 minor (low serum epo).
or 2 minor + 1 major.
complication: budd chair syndrome.
malignant transformation.
plebotomy, myelosuppresant -suppress bone marrow (use for all myeloprolfierative diseases)
(hydroxyurea), antihistamine, uric acid.

essential thrombocytosis

lots of platelets
lots of thrombosis, but platelets such so also get bleeds.
pt super high, large / immmature megakarytoces.
megakarytoes hypertrophy/hyperplasia.
dx of excusion: infection, inflammation, anemia, splenectomy, trauma, surgery, stress, ischemia.
bleeding time@: prolonged- suck
PT, PTT, normal -> coagulation factors normal.
malignant transformation->
hydroxyurea *cytoreductive therapy).

primary myelofibrosis- leading to BM firosis, hemopoesis, splenomegaly.
secondary: caused by other myeloprolfierative disorders (leukemias).
CF: weakness, weight, splenomegal
dx: CML, E, AML, MDS.
jaks inhibitor: rixolitinib.
supprotive,

treamtnet for PV

jaks 2 mutaiton-> tyrosine kinase.
onsidious onset. >60 years.

increase B12.
epo levels increased in 2 polythemia.
400 x 109 for platelet cont.
venesection: remove 500 ml weekly.
chemotherapy: hydroxycarbamide.
low aspirin-100 mg daily. recurrent thrombolytic epiosodes.

esential thrombocytopaeia:

myelodysplasia

hypercellular, dysplastic, pancytopneia,
aplastic anemia- low rbc (cancer).
mds< 20 %
pre-leukemic, trx to prevent MDS-> AML.
sideroblastic anemia caused via MDS (acquired colonial).
myeloid disorders -> AML,CML,MDS.
mds : inadequate maturation and cytopenia.
M>F. elderly.
Pancytopenia (all lines affected).
infection, bleeds, anemia/fatigue.
de novo mutaiton or environemtnal (benzen exposure), chemo/radiaiton).
30% cause (dysplasia s pre-cancerous)>
MDS: refractory -» ringed siderobalst.
chronic myelomonocyte.
thombocytosis / good prognosis.
unfavorable: percentage of blasts, cytopenia, age, cytogenetic abnormality.
CBC; low reticulocyte count, high mcv.
biopsy, peripheral smear- large aganular platelets, ringedsideroblastic.
dimoprhic rbc (rdw high).
dx: folate deficiency.
aplasitc anemia, myelofibrosis.

myelodysplastic syndromes trx:

supprotive, HGF (help them maturate), chemo or stell cell transplant.
no sx+ normal: no trx.
stem cell transplant-> cure.
epo, HGF, transfusion-> overload (deferoximine).
platelet trnasfusion.HGF.
epoetin, barbepoetin alpha.
platelts: elthrombopag.
wbc: G-CSF pegfilgrastim.
chemotherapy:
hypomethylating agent-
standard-cytarabine-> trash brain, hair loss.
immunmodulting: linalidomide (thalimodie -> teratogenic MM).
imunosuppresant -> cyclosporine (kidney)
se: GVHD (graft vs host).
peripheral blood smear: single/bilobed granulocytes.
kills bone marrow.

classification of MDS

1.refractory anaemia dysplastic changes, <1 % dysplastic blood, <5% in marrow.
2.refracotry anemia w/ ringed sideroblast. nucleated Er.
3. refractory anemia + excess blast. 1-5% in blood, more in marrow
4. w transformation. 20-3- myeloblasts
5. chronic myelomonocytic leukemia (CMML).> 1000 monocyte in blood & marrow
sx vaug, incidental finding.
dx: 5q, 22q deletion -cytogenetic.
qualitative and quantitively analysis-> abnormal.
bilobed nuclei, macrocytic anemia.

acute leukemia-AML, ALL

RF: geentic vs enviornment.
antineoplastic agent.
ionziing raidiation,hodkings lymphoma, MM, benzene.
monoclonal disorderAND ealry hemopoetic stem cell (large, immature cells).
ealry myeloid/early lymphoid-> replicate but cannot diffferentiate/useless.
replace most BM cells-> overcrowd normal hemaotpoesis- bleeds, infection, mucosal bleeds.
coagulation issue-> major bleeds.
enter peripheal blood- leukostasis.
metasise throughout body, headahce, impotent, bone/tender over sternum, splenomegaly.
CF: abrupt, younger, painless LN.
dx: perioehral bloods (large imature .
folic , acrocytic normocytic (rapid high turnove).
<100,00 for wbc and same for platelets.
morphology anlaysis, immunofloresxent, cell maekweea, cell markers, sudan black etc. stains.
MGF, induction phase (idncue chemotherPY-> supress all cell lines-> pancytopenia.
consolidation therapy: prlong emission, therapy (when its gone).
use gene expression to predic,t guide theraphy.
lymphoma for radiation.
SC TRANSPLANT.

acute leukemias

CF: onset young, sudden, fatigue, painless LN, t-cel deposit in skin, importnet and all causes headahce, hepatosplenomegaly.
dx: peripheral: normo or macrocytic anemia.
folic deficiency.
platelet: 150-400 normal but <100,000.
wbc:<10,00 or 100,000.
melod blast or lumhoblast (myelo/monoblast seen)
biopsy:>20% replaced completely. hypercellular.
kaytoype, moelcular marker, peroxidase detect it.
trx: induction (remission)-> high dose chemo.
infecton specially neutropenia.
myelid GF: reduce hospitalisatiin time.
give rbc and platelet infusions.
consolidation: normalise-> prolong rmeisision.
gene expresion-> predict prognosis
lymphoma - radiation. if youner transplant.

hodgkin lymphoma

solid tumor of immune system, LN, spleen, malt, bone marrow, thymus.
nonhodgkin more common..
more prognosis /better.
M>F>
bimodial age. 1-2 peak (young and older.
affect younger patient.
cervical lN, hiv higher risk, defect in cell mediated immunity.
benzene
hodkin->ALL.
EBV: mononucleous.
contagious-> spread locally.
extranodal (NHL).
lyadenopaty-> localised/painless.
trx with radiation-> NHL.
2nd amyloidosis (serum A amyloidosis).
classic: nodular slcerosis, lymphocyte rich, depleted, mixed.
nodlar lymphpcyte predominance (non classic).
nodular schelrosis (roses-> ffemale/children).
good prognosis. reed sternberg. lym
ant mediastinal nodes involved.
mixed cellularity-HIV< elderly, abdominal LN=psleen. more than 1 type of cell.
lymphocyte depleted-Least common. most aggressive.

genetic: NF-KB light chain. prolfieratin.
supraclavicular and antmediastinal.
bipsy: core needle. rubery bulging appearance. immunohistochemical.
cd30+ 15.
TTTT types.
fever on /off.
LN,itching
splenomegaly (not massive), nephortic -minimal change disease, jaudncie, erythea nodosa, anemia (autoimmune-> sen in CLL).
pain on alcohol consumption.
dx: neur.eosphinophilia.
poor prognosis if low. HIgh lDH-> poor prognosis.
CXR +CT staging.
pet scan (tumor shrink).
ann arbor classification: single site, > 2 LN,
both sides, diffuse and extralympatic tissue.
B-B symtpoms, might sweat, fatigue and wt loss. 10%.
ABVD: adriamyicn, belomycin, vinblasrine, dacarbazine.
radiotherapy + chemo. (higher stag,e more cycles)>
comlication: hypothryoidism.

non hodgkin

nodal and extra ndoal. constituional and hepatosplenomegaly. common for b-cells.
aggresive and indolent subtypes.
itching + bsymtpoms.
associated with aids, EBv etc.compression of nearby strucutres.
cytopena autoimune and low igM.
immunosuppresion causes.
median age- 50 years.
viruses: EBV, hep C.
radiation. h.pylori.
dx: excisional biopsy.
immunohistology, measure igM/igG.
uric acid (High).
physical and ct -TAP.
pet scan: monitor.
cyopenia- boen marrow to determine cause.
aggressive vs indolent:
h.pylori- gastric/maltoma.
HCv-> b-cell lymphoma.
sjjoren-Salvary lymoma.
supvena syndrome.
has leukemic phase-> see in blood (spread to bone marrow)>

high grade-> young, chemo to cure, bad prognosis.
low grade: older, sensive, and aggressive change
aggressive: burkitt, diffuse b-cell, mantle cell.
indolent -follicular, hairy cell leukemia.

CLL

mature, funcitolaly imcompetent- lymphoprolfierative.
solid nodal mass.
eukemia-> lymphoma.
asx.
newborn -ALL
40-60-AML/CML.
>60 - CLL.
smudge cells lymphoctois, lympahdenopathy, hepatosplenomegaly.
anemia, thrombocytopenoa (poor prognosis).
stage 0- lymphocyto.
1alymphadenoenopahty, splenomegaly.
2anemia
3-thrombocytopnenia
4
rai system.
10% difuse large cell lymhpoma (aggressive)
cytopenia.

multiple myeloma

monoclonal gammopathie-> dysproteinemia.
gammma antibody chains globulin.
B-lypho-> plasma cells-> Ab.
most cases, light chain in excess-> cleared via kidney.
small<10 mg /day.
only in electorphoresis. not disptick -> only detect albumin.
bence johns protein.
M-spike (gamma protein increase).
use immunoelectrophoresis (imunofixation) to distinguish type of gamma chain in excess.
singular igGigA etc .
crowd out other antibody-> immunodeficiency since others are deficient.
risk of infection (virus not prone since t-cell intact).
paraproteins.
older age.
benzene/
pathogenesis-> dysrgulation of clyclin D- boe arrow->il6-> myeloma.
osteoclast and osteoblast inhibited-> bone lesions.
ALP (no osteoblast) normal.
nuclear bone scan normal. x-ray detected only.
normal_>MGUS->MM.
bone pain (ribs, back, skull etc0>
punched out lesionns, hypercalcemia, weak bones.
hematolgoy: normocytic , normochromatic anemia.
high esr.
bleeding time, platelet deficiency (cover platelets).
roulex formation (clump-> ink) mproteins.
dialysis corrects bleeds.
interact with coauglation factors., amylodioss damnges endothelium-> clots.
radiculopahty(root of nerve), neuropahty-> bladder+ bowel control loss.
lethary, confsion, weakness, hyperviscosity syndrome kidney failure.
CRAB: calcium renal infussiency, anemia, bone ppain/
fanconi syndrome.
MM-ALL.
trx: rehydrate, nalgesia, local radioterapy.abx for bacteria infection#
VAD regmen-Dexamethasone, vincrisitne adriamyic.

HSP

igA vasculitis.
children-lower leg, vuttock.
kidney, GIT (abdo pain->hemorhage, ifnarction)
triggered vi upper RTI< gastroenteritis. <10 yrs/.
kidney impairemnet.
leaky SV bleeds-> purple red lumps-> non blanching.
features: 100%.
affect arms/ trunk. necrosis if severe.
arhtalgaarthritis.
iga nephirtis-> hematuria.
proteinuria
2+ protein urinalysis-> nephrotic.
ddx: leuekmia,thrombocytopie ourpura, HUS.
investigation: CBC, film (sesis, leukemia, sepsis0< labuminrenal profile, urine dipstick, BUN, BP check (HTN).

palpble purpuea and other sx.
management: analgesia, rest , hydration. maybe steroids.
monitor closely

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